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Published on: August 8, 2022
Clinical application of WHF-MOGE(S) classification for hypertrophic cardiomyopathy
Anushree Agarwal1, Rayan Yousefzai1, M Fuad Jan1
1Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke's Medical Centers, University of Wisconsin School of Medicine and Public Health, Milwaukee, WI, USA.
Insights
The MOGE(S) classification system was applied to hypertrophic cardiomyopathy (HCM) patients. Genotype-positive HCM patients showed distinct clinical features, suggesting a need for classification refinement.
Area of Science:
- Cardiology and Genetic Medicine
- Cardiovascular Research
- Clinical Genetics
Background:
- The Morphofunctional, Organ involvement, Genetics, Etiology, and functional Status (MOGE(S)) system is a novel nosology for classifying cardiomyopathies.
- The clinical utility of the MOGE(S) system in hypertrophic cardiomyopathy (HCM) has not been previously documented.
Purpose of the Study:
- To conduct a comprehensive genotypic and phenotypic evaluation of hypertrophic cardiomyopathy (HCM) patients using the MOGE(S) classification.
- To assess the clinical applicability and refine the MOGE(S) classification for HCM.
Main Methods:
- Evaluation of 254 patients (190 probands, 64 family members) with HCM from January 2011 to March 2014.
- Analysis of clinical, imaging, and follow-up data, including gene testing for 129 patients.
- Categorization of patients into gene-positive (MHOHGADEG+) and gene-negative (MHOHGADEG-) groups based on MOGE(S) criteria.
Main Results:
- 181 patients were diagnosed with HCM phenotype, with 54.7% males and mean maximal left ventricular thickness of 2.2 cm.
- Obstructive HCM was identified in 66.3% of patients, with an average peak gradient of 57.1.
- Genotype-positive (MHOHGADEG+) patients were younger, more likely female, had a family history of HCM/sudden death, lower gradients, and more sudden death risk factors.
Conclusions:
- The MOGE(S) classification provides a framework for describing HCM genotype-phenotype correlations.
- Genotype-positive HCM patients exhibit distinct clinical characteristics compared to gene-negative individuals.
- A proposed modification to the MOGE(S) classification for HCM is suggested, incorporating obstruction and hypertrophy location.
Background:
Recently, a new MOGE(S) (Morphofunctional, Organ involvement, Genetics, Etiology of details of the genetic disease or underlying cause, and functional Status) genotype to phenotype nosology system for classification of cardiomyopathies was proposed, but its clinical use has not been described.
Objectives:
This study presents the comprehensive geno-phenotypic evaluation of hypertrophic cardiomyopathy (HCM) patients by employing the newly proposed World Heart Federation classification of cardiomyopathies - the MOGE(S) classification.
Methods:
From January 2011 to March 2014, 254 patients were evaluated (190 probands and 64 family members). Of those, 181 were HCM phenotype-positive probands, and 54.7% were male patients. Mean maximal left ventricular thickness was 2.2 ± 0.6 cm, with >2.5 cm thickness seen in 21.5% of patients. Obstructive HCM was present in 66.3% of patients, with an average peak gradient of 57.1 ± 47.2. Detailed clinical, imaging, and follow-up data were analyzed. Gene testing was performed in 129 patients (67.9%), and they were categorized into gene-positive (MHOHGADEG+) and gene-negative (MHOHGADEG-) groups based on the MOGE(S) classification.
Results:
MHOHGADEG+ patients were younger at time of diagnosis, more likely to be female, more likely to have ventricular tachycardia and a family history of HCM or sudden death, had lower peak gradients, and were more likely to have sudden death risk factors.
Conclusions:
In addition to employing genotype-to-phenotype nosology to describe HCM, we propose a modification to the current MOGE(S) classification for HCM based on the presence or absence of obstruction and location of hypertrophy within the morphology.
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